Intracranial hemorrhage (ICH) is a rare but devastating complication of childhood immune thrombocytopenia purpura (ITP). A survey of ICH from 1987 to 2000 identified cases of ICH in childhood ITP in the United States. Forty patients with ICH and 80 matched ITP control subjects were accrued. The estimated incidence of ICH was 0.19% to 0.78%. Platelet counts were less than 20 ؋ 10 9 /L in 90% and less than 10 ؋ 10 9 /L in 75% of children with ICH. Eighteen (45%) children developed ICH within 7 days of diagnosis of ITP; for 10 of these, ICH was the presenting feature of ITP. Twelve (30%) children had chronic ITP. Head trauma and hematuria were the most prominent features associated with ICH, identified in 33% and 22.5% of the patients with ICH and 1 and none of the controls (both P < .001). Bleeding beyond petechiae and ecchymoses was also linked to ICH. Mortality was 25%; a further 25% had neurologic sequelae. Strategies by which high-risk children could be identified were considered, and the costs of preventive combination treatment were estimated. Children with severe thrombocytopenia plus head trauma and/or hematuria appeared to be at particularly high risk of ICH. Aggressive treatment of these children may be appropriate. (Blood. 2009;114:4777-4783)

This pilot study used an immune thrombocytopenic purpura (ITP)-specific bleeding score, the ITP Bleeding Scale (IBLS) to analyse the correlation of clinical and laboratory platelet variables with bleeding. MethodsA prospective Institutional Review Board-approved study was conducted on 100 visits for 65 consenting patients with ITP (30 on one and 35 on two visits) from December 2004 to December 2005. The patients were primarily adults with chronic ITP; a minority were children or adults with acute ITP.The IBLS (Table I) comprised 11 grades from 0 (none) to 2 (marked bleeding) assessed at nine anatomical sites by history over the previous week (Hx). In addition, two of these sites, skin and oral, were also assessed by physical examination (PE). The 'worst ever' bleeding experienced at each site was graded using the same system. Blood counts were analysed by using the Bayer-ADVIA TM 120 (Giacomini et al, 2001). Large platelets (20-60 fl) were examined as they were reported to be more haemostatically active (Karpatkin, 1978;Michel et al, 2005). Analysis focused on the six grades with most bleeding -skin and oral (Hx and PE), epistaxis and gynaecological (GYN) (Hx). Insufficient haemorrhages occurred at the other sites to enable inclusion in the analysis. Means, medians, ranges, the Kruskal-Wallis, Fisher's exact and chi-squared tests, and the Kappa statistic to establish inter-observer reliability (for 63/100 visits) were calculated. P-values <0AE05 were considered significant. ResultsThe median age was 31AE5 years; 20 patients were under 18 and eight over 65 years. Forty-two were female, 23 of reproductive age and assessed for GYN bleeding on 36 visits. Eighty-eight per cent had chronic ITP (median duration 6 years) and 40% were splenectomised. No correlation between IBLS and age, sex, duration of ITP or splenectomy status was found.The IBLS grades on the 100 study visits are presented in Tables II-IV. Seventy-two per cent of grade 1 haemorrhages occured in the skin. Grade 2 bleeding was more heterogeneous (28% skin, 46% oral, 13% GYN and 10% epistaxis). Patients with more skin bleeding also had more oral bleeding (PE and Hx, all P-values <0AE026).The median platelet count for all visits was 39AE5 · 10 9 /l (range: 6-623 · 10 9 /l). Twenty-five visits had a platelet count £ 20 · 10 9 /l and 46 visits had a platelet SummaryA method for objective quantification of bleeding symptoms in immune thrombocytopenic purpura (ITP) has not been established. The ITP Bleeding Scale (IBLS) is a novel bleeding assessment system comprising 11 site-specific grades. Implementation of the IBLS on 100 patient visits revealed that although platelet count and large platelet count correlated well with bleeding symptoms overall, this relationship disappeared in marked thrombocytopenia. The IBLS is a useful clinical tool for monitoring bleeding and may be used to aid the development of laboratory parameters that correlate with underlying bleeding propensity in thrombocytopenia.